Chronic granulomatous disease. Update and review

Abstract

Chronic granulomatous disease (CGD) is a primary immunodeficiency syndrome which is characterized by increased susceptibility to severe fungal and bacterial infections. CGD is the result of the lack of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase enzyme in the patient’s phagocytes to produce superoxide. It is characterized by recurrent infections with a narrow spectrum of bacteria and fungi, as well as a common set of inflammatory complications, including inflammatory bowel disease. The most frequently found pathogens are Staphylococcus aureus, species of Aspergillus, species of Klebsiella, Burkholderia cepacia, Serratia marcescens and species of Salmonella. Long term antibiotic prophylaxis has helped fight infections associated with chronic granulomatous disease, while the steady progress in bone marrow transplants and the possibility of gene therapy are defined as permanent treatment options.